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70-8-第八节 预后以及活动评分
一、预后( S! ] e. E0 k" M' B7 c7 {
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韦格纳肉芽肿通过用药尤其是糖皮质激素加环磷酞胺联合治疗和严密的随诊,能诱导和维持长期的缓解。早期诊断能预期获得有效的治疗。最近几年,在疾病早期即可获得韦格纳肉芽肿的诊断,使患者的治疗效果更好并得到缓解。过去,未经治疗的韦格纳肉芽肿平均生存期是 5个月,82%的患者一年内死亡,约90%的患者两年内死亡。目前经激素和免疫抑制剂治疗后,WG的预后明显改善,大部分患者在正确治疗下能维持长期缓解。1992年,Hoff-man统计的8年死亡率为13%,1996年,Mat-teson公布的5年和10年死亡率分别为28%和36 。影响预后的主要因素是难以控制的感染和不可逆的肾脏损害,年龄大于57岁及血肌醉升高是预后不良因素。此外,ANCA的类型对治疗的反应和预后似乎无关,但有抗PR3抗体的患者若不治疗有可能病情更活动,进展更迅速。故早期诊断、早期治疗,力争在肾功能损害之前给予积极治疗,可明显改善预后。
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# E- A7 d3 t; J 二、缓解的定义 ]! R2 W0 V4 G: }% V
: J" Q) s% q' q+ B e, a, K 韦格纳肉芽肿是否缓解取决于其炎症是否活动,而不是一些功能检查的异常,患者的临床表现异常可能并非是疾病活动。例如,一位患者的系统性的症状和体征已解决,尿液分析已不活动,那么即使有持续的肾功能不全,仍认为已缓解。这种持续的损害被看成是疾病活动期导致的不可逆的损害。当然以后肾病的进展也许是由于其他因素引起而非疾病活动。$ A4 T) I4 j9 c+ w
三、活动评分
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# a5 U! G' H( p+ V ` 制定活动评分是为了评估病情活动和预后,指导治疗,本文介绍的评分标准是根据伯明翰系统性坏死性血管炎评分标准修订的WG标准 (BVAS/WG)见表 70一5。与 BVAS相比,BVAS/WG取消了38个条目,修改了9个条目,新增了7个条目。BVAS详见血管炎总论章节。
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参考文献;* R1 k, i/ Z; V/ p; h2 f: I' {
# o# C, S) g2 K0 @: ^8 [' m9 w BacchettaR, Sartirana C, Levings MK, et al. Growth andexpansion of human T regulatory type 1 cells are independentfrom TCR activation but require exogenous cytokines. Eur J Im-munol,2002.32:2237一2245 3 R+ R! {, r* `) J$ [
' R& o+ c' d h3 J" H% o" Q
Calabrese LH, Duna G. Vasculitis associated with antineu-trophil cytoplasmic antibody. In: Shaun Ruddy ed. Kelley’sTextbook of Rheumatology, 6th Ed. Philadelphia: W. B. Saun-ders Company, 2001.1165一1184
1 C3 c! ~1 {$ w! M Cameron SB, Nawijn MC, Kum WW, et al. Regulation ofhelper T cell responses to staphylococcal superantigens. Eur Cy-tokine Netw,2001.12:210一222
. |# P% U* p6 M, _* e( q" ^7 y! `- b/ {7 R8 w0 j
Cordier JF, Valeyre D, Guillevin L. Pulmonary Wegener' sgranulomatosis. A clinical and imaging study of 77 cases. Chest,1990.97:906一912
7 v3 A8 Y4 Q- l4 v$ b( b) A6 r$ o4 v- B; y9 u& p5 A
Dieckmann D, Bruett CH, Ploetmer H, et al. Human CD4(+)CD25(+)regulatory, contact-dependent T cells induce in-terleukinT cells. J1-producing, contact-independent type 1-like regulatoryDunaExp Med,2002.196:247一253$ Y: l" v6 b3 A! T1 W
5 [2 G! @1 ?0 l# p& ^7 E B. F GF, Galperin C, Hoffman GS. Wegener' s granulo-matosis. Rheum Dis Clin North Am, 1995.21:949一986
; M J1 |: k: c. W: }% D Frazier AA,Rosado-de-Christenson ML, Galvin JR, et al.Pulmonary angiitis and granulomatosis: radiologic-pathologic cor-relation. Radiographics,1998. 18:687一710
1 }0 u) I+ V. b R, _: _! B
0 N$ B+ W; C% v- f Guillevin L, Cordier J, Lhote F, et al. A prospective, mul-ticenter, randomized trial comparing steroids and pulse cyclophos-phamide versus steroids and oral cyclophosphamide in the treat-ment of generalized Wegener's granulomatosis. Arthritis Rheum,1997. 40:2187一2198
5 Q- }: O! A/ b5 ]3 u( M9 L+ d2 o: L3 z5 l9 ]- ?$ o
Hagen EC, Daha MR, Hermans J, et al. Diagnostic value ofstandardized assays for anti-neutrophil cytoplasmic antibodies inidiopathic systemic vasculitis. EC/BCR Project for ANCA AssayStandardization. Kidney Int,1998. 53:743一753
9 d" N: Q) j Z) h' |) a; e# [5 d5 e! R" u+ G6 k
Haubitz M, Koch KM, Brunkhorst R. Survival and vasculi-tis activity in patients with end-stage renal disease due to Wegen-er's granulomatosis. Nephrol Dial Transplant, 1998. 13: 1713-1718
0 `- H. e5 q6 B4 h3 ?5 S) C2 U, O% f& _3 N' W
Hoffman GS. Treatment of Wegener's granulomatosis: Timeto change the standard of care? Arthritis Rheum, 1997. 40: 2099一2104 : J- u9 J4 x6 z5 X. ^
7 T7 n4 X# c; k/ S0 u, H0 l+ V Lie JT. Nomenclature and classification of vasculitis: Plus cachange, plus c' est la meme chose. Arthritis Rheum, 1994. 37:181一186 : S* e) N% }/ g$ c5 g8 `% y
5 c$ q: Z+ p8 d* ], W
Reinhold-Keller E, Beuge N, Latza U, et al. An interdisci-plinary approachto the care of patients with Wegener's granulo-matosis: long-term outcome in 155 patients. Arthritis Rheum,2000.43:1021一1032 6 K) q5 b9 x. y2 e
# x/ W1 O1 e2 [: {! e" ~8 I! W Rojo-Leyva F, Ratliff NB, Cosgrove DM. Study of 52 pa-tients with idiopathic aortitis from a cohort of 1 204 surgical cas-es. Arthritis Rheum, 2000.43:901一907
+ \8 V% i" Z+ d6 m& z' y- k5 u% p I' \( o0 M
Savige J,Davies D, Falk用,et al. Antineutrophil cytoplas-mic antibodies and associated diseases: a review of the clinical andlaboratory features. Kidney Int, 2000.57:846一862
( Z- x5 y5 k1 ]* a q* {7 J: T( a; a, r) \
Stone JH, Hoffman GS, Merkel PA, et al. A disease-specificactivity index for Wegener's granulomatosis. Modification of theBirmingham Vasculitis Activity Score. Arthritis Rheum, 2001.44:912一920 |
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